Primary Sclerosing Epithelioid Fibrosarcoma of the Kidney: A Case Report and Review of the Literature

被引：0

作者：

Bravo-Taxa, Mercedes ^{[1
]}

Garatea-Grau, Rafael ^{[2
]}

Nunez-Romero, Ulises ^{[2
]}

Huanca-Amesquita, Lourdes ^{[1
,3
]}

机构：

[1] Inst Nacl Enfermedades Neoplas INEN, Pathol Dept, Lima, Peru

[2] Clin Ricardo Palma, Surg Dept, Lima, Peru

[3] Clin Ricardo Palma, Pathol Dept, Lima, Peru

来源：

AJSP-REVIEWS AND REPORTS | 2022年 / 27卷 / 01期

关键词：

EWSR1; fibrosarcoma; kidney; MUC4; sclerosing epithelioid sarcoma; SARCOMA; MARKER; MUC4;

D O I：

10.1097/PCR.0000000000000483

中图分类号：

R36 [病理学];

学科分类号：

100104 ;

摘要：

Sclerosing epithelioid fibrosarcoma (SEF) is a rare fibrosarcoma variant that features rounded carcinoma-like epithelioid cells arranged into compact nests, cords, and single-cell patterns within a highly sclerotic stroma and has a consistent translocation (EWSR1-CREB3L1/2). To our knowledge, there are 110 cases of pure SEF reported, to date, with 15 occurring at intra-abdominal sites. Primary SEF of the kidney is exceptionally rare. We present a case of SEF that arose in the kidney. Histological examination revealed densely hyalinized epithelioid tumor suggestive of SEF. The diffuse immunohistochemical staining of MUC4 by neoplastic cells and the presence of EWSR1 gene rearrangement by fluorescence in situ hybridization analysis confirmed the histological diagnosis.

引用

页码：13 / 17

页数：5

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